DDW: Study Reveals Clues to Lymphoma In Celiac Patients

Action Points

Note that this study was published as an abstract and presented at a conference. These data and conclusions should be considered to be preliminary until published in a peer-reviewed journal.

Explain that certain subgroups of patients with celiac disease have characteristics that put them at a higher risk for a lymphoproliferative disorder, including older age or presence of symptoms (abdominal pain, diarrhea)﻿ at diagnosis.

CHICAGO -- Certain subgroups of patients with celiac disease, such as those who develop this autoimmune disease in older middle age, also are at higher risk for lymphoproliferative disorders, a researcher said here.

Patients who developed lymphoproliferative disorders were older when their celiac disease was diagnosed (55 versus 43 years, P<0.0001), according to Lori A. Leslie and colleagues from Columbia University in New York City.

In addition, they were more likely to present with symptoms such as abdominal pain (12% versus 3%, P=0.041) and diarrhea (69% versus 40%, P=0.0068), Leslie reported at Digestive Disease Week.

Patients with celiac disease have an increased incidence of lymphoma, and recent reports have suggested that this is on the rise.

However, little is known about the incidence of the various subtypes of lymphoproliferative disorders in these patients.

"It's important to differentiate between these subtypes because there are major differences in prognosis and treatment," Leslie said.

To explore this, she and her colleagues conducted a retrospective study of 1,281 patients treated for celiac disease at Columbia during the past 30 years, identifying 35 cases of the various lymphoproliferative disorders.

To compare the incidence of these disorders with the incidence in the general population, they determined the expected incidence using the Surveillance, Epidemiology, and End Result (SEER) database.

There were 29 cases of non-Hodgkin's lymphoma, one Hodgkin's lymphoma, and five cases of chronic lymphocytic leukemia.

The B-cell lymphomas were further characterized as four diffuse large-B cell, three mantle cell, four marginal zone, one follicular, one primary effusion, and one case of post-transplantation lymphoproliferative disorder.

Standardized incidence ratios for the specific subtypes were:

All lymphoproliferative disorders, 5.7 (95% CI 3.9 to 7.7)

Non-Hodgkin's lymphoma, 3.4 (95% CI 1.9 to 5)

Chronic lymphocytic leukemia, 4.1 (95% CI 1.2 to 8.3)

Peripheral T-cell, 18 (95% CI 4.6-39.9)

Diffuse large B-cell, 3.6 (95% CI 0.9 to 7.9)

Mantle cell, 32.3 (95% CI 6 to 79.1)

Marginal zone, 29.8 (95% CI 7.7 to 66.2)

No standardized incidence ratio could be calculated for enteropathy-associated T-cell lymphoma because the expected incidence was zero.

"But the incidence was notably elevated in this cohort," Leslie said.

A total of 70% of the celiac disease patients were women, which is in line with the recognized female predominance of this disease. Yet the incidence of non-Hodgkin's lymphoma was similar for women (6, 95% CI 3.7 to 8.9) and men (5.3, 95% CI 2.9 to 8.4).

In 20 cases the celiac disease was diagnosed before the lymphoma by a mean of 7.8 years, and in ten cases the celiac disease was diagnosed later by a mean of 6.6 years.

In the remaining five, the two diseases were diagnosed concurrently.

"Patients diagnosed with celiac disease after age 50, who initially present with diarrhea and/or abdominal pain, are at increased risk and may need routine follow up and surveillance for the development of lymphoma," Leslie concluded.

Three of the co-authors disclosed consulting for Shire Pharmaceuticals or Boehringer Ingelheim, acting on advisory committees for Executive Health Exams, Genentech, Alvine, and Alba Therapeutics, and receiving research support from Salix Pharmaceuticals.